{Reference Type}: Journal Article
{Title}: Intracranial Phosphaturic Mesenchymal Tumors: A Systematic Literature Review of a Rare Entity.
{Author}: Sánchez Muñoz R;Sanmillan JL;Vidal Sarró N;Martín Quintero G;Martí Duce D;Gabarrós Canals A;
{Journal}: World Neurosurg
{Volume}: 187
{Issue}: 0
{Year}: 2024 Jul 30
{Factor}: 2.21
{DOI}: 10.1016/j.wneu.2024.03.138
{Abstract}: <B>BACKGROUND: </B>Phosphaturic Mesenchymal Tumors (PMTs) are rare mesenchymal neoplasms known for producing Tumor-induced Osteomalacia (TIO). TIO is an uncommon paraneoplastic syndrome characterized by radiographic evidence of inadequate bone mineralization and analytical abnormalites.<BR><B>METHODS: </B>We sought to present a case of TIO caused by skull base PMT with intracranial extension, manifesting with pain, progressive weakness, and multiple bone fractures. Furthermore, a systematic review was performed, following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. A search was conducted in PubMed database with title/abstract keywords "Phosphaturic mesenchymal tumor" and "Osteomalacia." Search results were reviewed looking for intracranial or skull base tumors.<BR><B>RESULTS: </B>Our systematic review included 29 reported cases of intracranial PMT. In the reviewed cases there was a significative female predominance with 22 cases (75,86%). Osteomalacia was presented in 25 cases (86,20%). Bone fractures were present in 10 cases (34,48%). The most common site of involvement was the anterior cranial fossa in 14 cases (48,27%). Surgery was performed in 27 cases (93,10%) with previous tumor embolization in 4 cases (13,79%). Total recovery of the presenting symptoms in the first year was achieved in 21 cases (72,41%). Recurrence of the disease was described in 6 cases (25%).<BR><B>CONCLUSIONS: </B>Skull base PMTs with intracranial extension are extremely rare tumors. Most patients are middle-aged adults with a PMT predominantly located in anterior cranial fossa. Surgery is the current treatment of choice with optimal outcome at 1-year follow-up, although recurrence could be present in almost 25% of the cases.